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Pulmonary veno‐occlusive disease in an 11‐year‐old girl: Diagnostic pitfalls
Author(s) -
Kano Gen,
Nakamura Keiko,
Sakamoto Izumi
Publication year - 2014
Publication title -
pediatrics international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.49
H-Index - 63
eISSN - 1442-200X
pISSN - 1328-8067
DOI - 10.1111/ped.12237
Subject(s) - medicine , pulmonary veno occlusive disease , lung transplantation , hemosiderosis , lung , disease , lung biopsy , prednisolone , surgery , transplantation , pathology
Pulmonary veno‐occlusive disease ( PVOD ) is a rare chronic lung disease that is difficult to diagnose due to non‐specific clinical findings. Little is known about the pathogenesis of PVOD . Reported herein is the case of an 11‐year‐old girl who initially presented with ‘bat‐wing’ shadows on chest radiography. This finding, coupled with prominent hemosiderosis in bronchoalveolar lavage fluid, initially led to a misdiagnosis of idiopathic pulmonary hemosiderosis. Oral prednisolone dramatically improved signs and symptoms initially, but her condition then gradually deteriorated during maintenance therapy with corticosteroids and other immunosuppressants. PVOD was suspected but not confirmed owing to a lack of hallmark radiographic findings and contraindications for lung biopsy. Three years later, while arranging for lung transplantation, the patient experienced sudden onset of fatal massive pulmonary edema. PVOD was confirmed at autopsy. This case provides insights regarding an unfamiliar presentation of PVOD and may help physicians to avoid diagnostic pitfalls.